Abstract: Fabry's disease is an X-linked inherited lysosomal storage disorder caused by the deficient activity of alpha-galactosidase A. The interrelationships between clinical symptoms in Fabry patients have not yet been fully established. Using cluster and multivariate analysis, the aim of the study was to determine the relationships among clinical symptoms and organ involvement, and predictive clinical symptoms for disease severity.
Abstract: Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory ocular disease (mainly interstitial keratitis) and audiovestibular symptoms (mainly acute-onset sensorineural hearing loss) in the setting of a negative work-up for syphilis. The diversity of the ocular and audiovestibular manifestations reported in the literature in atypical Cogan's syndrome should make one cautious before accepting the diagnosis as the disease may mimic various other systemic disorders. Systemic corticosteroids are always the most widely used and successful therapy. For patients requiring high and prolonged doses, additional immunosuppression is appropriate. Methotrexate is the first-line steroid sparing agent. However, patients without systemic disease or severe eye disease unmanageable by topical corticosteroids should not be subjected to protracted courses of corticosteroids or immunosuppressive agents, particularly when little gain in hearing is obtained with their use. The effect of TNF-alpha blockers was recently investigated. Infliximab might be an alternative therapy in cases of failure of corticosteroids and immunosuppressive therapy. However, treatment might be more effective when started at an early stage of the disease, when the lesions are still reversible.
Abstract: Physicians need to be familiar with the typical manifestations of giant cell arteritis. However, the challenge lies in recognizing atypical cases that lack the more specific manifestations or reflect vasculitis in less frequently involved territories. Among atypical clinical manifestations, dry cough has been reported in recent years. The literature contains sporadic reports mainly single case report. The objective of this study was to determine the frequency of dry cough in patients with giant cell arteritis. Clinical data were collected from 88 patients with giant cell arteritis. Relationships between dry cough and other clinical manifestations or biological data were analyzed. Dry cough of recent appearance was found at initial presentation of giant cell arteritis in 12 patients (13.6 %). In 2 cases, dry cough was isolated. The 2 patients sought attention because of chronic dry cough associated with inflammation of unknown origin. In 10 cases, dry cough was associated with typical clinical manifestations of giant cell arteritis. A correlation was found between inflammatory biomarkers and presence of dry cough. The mean CRP was 153.8 mg/l (SD 85.1) in patients with dry cough and 94 mg/l (SD 72.2) in patients without dry cough (p = 0.0131). We conclude that the diagnosis of giant cell arteritis should always be considered in an elderly patient with an unexplained elevation of inflammatory markers and chronic dry cough. Dry cough in giant cell arteritis was not correlated with other clinical manifestations of this vasculitis, including pulmonary manifestations, but was correlated with inflammatory biomarkers.
Abstract: Data on the clinical spectrum and therapeutic management of noninfectious mixed cryoglobulinemia vasculitis (CryoVas) in the era of hepatitis C virus screening are lacking. We analyzed data from 242 patients with noninfectious mixed CryoVas included in the French multicenter CryoVas survey. Baseline manifestations were purpura (75%), peripheral neuropathy (52%), arthralgia or arthritis (44%), glomerulonephritis (35%), cutaneous ulcers (16%), and cutaneous necrosis (14%). A connective tissue disease was diagnosed in 30% and B-cell non-Hodgkin lymphoma in 22%, whereas the CryoVas was considered to be essential in 48%. With the use of Cox-marginal structural models, rituximab plus corticosteroids showed the greater therapeutic efficacy compared with corticosteroids alone and alkylating agents plus corticosteroids to achieve complete clinical, renal, and immunologic responses and a prednisone dosage < 10 mg/d at 6 months. However, this regimen was also associated with severe infections, particularly when high doses of corticosteroids were used, whereas death rates did not differ between the therapeutic regimens. The role of each of these strategies remains to be defined in well-designed randomized controlled trials.
Abstract: Camptocormia, an abnormal truncal flexion posture that occurs while walking or standing, is usually caused by various hypokinetic movement disorders, mainly Parkinson disease. We describe the case of a man with subacute onset of camptocormia. Quadriceps muscle biopsy showed significant rhabdomyolysis, few isolated inflammatory cells and mild expression of type I MHC in few fibers, a pattern usually found in immune-mediated necrotizing myopathies. Myositis was associated with Raynaud's phenomenon, mild sclerodactyly, and anti-Ku antibodies leading to the diagnosis of polymyositis/systemic sclerosis overlap myositis. The posture showed modest improvement in response to treatment.
Abstract: The risk of malignant non-Hodgkin lymphoma is increased in primary Sjögren syndrome. In the literature, most studies evaluating this risk were conducted in tertiary reference university hospital. So, selection bias in series exists, in particular selection of the most severe cases in tertiary reference university care centers. Some studies had also a selection bias because patients were hospitalized (data were obtained from hospital discharge registries) and therefore the more severe cases were considered. Between October 1999 and November 2009, 109 adult patients were admitted to our department of internal medicine (non-university hospital, secondary level of the healthcare system, hospitalized patients and outpatient) with diagnosis of primary Sjögren syndrome. Two cases of parotid gland lymphoma occurred during the period of follow-up. No other lymphoma was detected. In this study, clinically identifiable parotid gland non-Hodgkin lymphoma occurs in 1.8% of patients with primary Sjögren syndrome. Because most non-Hodgkin lymphoma initially involves the neck organs, meticulous imaging studies mainly focused on the cervical regions are recommended in the follow-up of patients with primary Sjögren syndrome. Patients whose main complaint is persistent parotid gland swelling may have a parotid biopsy in order to diagnose non-Hodgkin lymphoma.
Abstract: Several epidemiologic studies suggest that Fabry disease should be considered in young patients with cryptogenic stroke. We report a case of a young woman presenting with recurrent ischemic strokes who was finally diagnosed with Fabry disease after impaired kidney function had been identified. Fabry disease should be considered in unexplained cases of first or recurrent strokes in young patients disregarding the gender of the patient, especially when chronic kidney disease and/or proteinuria are present. Renal function should be closely monitored in patients with strokes and followed up after the event. In this case, intravenous thrombolysis was performed after the second ischemic event. No other case of thrombolysis for ischemic stroke in Fabry disease has been described in the literature.
Abstract: Systemic clinical presentations of infection caused by Bartonella henselae are rare in immunocompetent adults. We report four cases with hepatic and/or splenic involvement, presenting as fever of unknown origin. We discuss diagnosis and treatment of this infection. Bartonella henselae serology allows an easy diagnosis of hepatosplenic involvement in cat scratch disease, a clinical picture that appears to be underrecognized.
Abstract: Management of nonviral cryoglobulinemia vasculitis has yet to be defined. Rituximab has emerged as a novel and promising therapeutic alternative, but data are scarce. Our objective was to evaluate the safety and efficacy of rituximab in nonviral cryoglobulinemia vasculitis in off-trial real-life patients.
Abstract: To determine the prevalence of and independent factors associated with joint involvement in a large population of patients with systemic sclerosis (SSc).
Abstract: Intoxication by glycyrrhizic acid is a well-known cause of the apparent mineralocorticoid excess syndrome. It causes hypokalemia by inhibiting the enzyme 11-beta-hydroxysteroid dehydrogenase. We report a case of hypokalemic rhabdomyolysis secondary to chronic glycyrrhizic acid intoxication.
Abstract: Hypocomplementemic urticarial vasculitis is a rare disorder characterized by the presence of C1q precipitins associated with a syndrome of urticarial vasculitis, arthralgias, ocular inflammation and obstructive-lung disease. We report the case of a 48-year-old woman with hypocomplementemic urticarial vasculitis. Because of dependance to corticosteroids, cyclophosphamide-pulse therapy was started and resulted in significant clinical improvement. Mycophenolate mofetil was effective as maintenance therapy and resulted in complete resolution of rash, arthralgias, arthritis and uveitis, but had no effect on the obstructive-lung disease.
Abstract: Noninfectious inflammatory diseases (connective tissue diseases, vasculitis syndromes, granulomatous diseases) emerged as the most frequent cause of fever of unknown origin in western countries. Among these diseases, giant cell arteritis and polymyalgia rheumatica are the most frequent specific diagnosis in the elderly and adult-onset Still's disease the most frequent in younger patients. This article focuses on noninfectious inflammatory diseases as a cause of classic fever of unknown origin (mainly rheumatic diseases, such as vasculitis and connective tissue diseases).
Abstract: BACKGROUND: We report a case of myelopathy during the course of lupus profundus that is unique to our knowledge. CASE-REPORT: A 29-year-old woman had lupus profundus since 1999, initially associated with thrombopenia (28,000 platelets/mm3) treated with corticosteroids for 6 months. Several nodular eruptions occurred from 1999 to 2004. Antinuclear antibodies were positive at 1/320 to 1/640 and complement C4 fraction was low. While being treated with hydroxychloroquine for a recent flare-up of nodular lesions of lupus profundus, she complained of paraesthesia of the abdominal wall and of the upper and lower limbs, suggestive of a medullary lesion. MRI disclosed an image of acute myelitis at the level of the second cervical vertebra. High doses of corticosteroids were promptly administered intravenously (methylprednisolone bolus) followed by oral prednisone and hydroxychloroquine. Six months later the patient was free of neurologic or cutaneous symptoms. DISCUSSION: In the literature, systemic signs are rarely associated with lupus profundus and myelitis has never been reported. Lupus myelitis is a vascular and/or demyelinating and usually segmental lesion of the spinal cord. Neurologic symptoms are those of acute rather than chronic or recurrent transverse myelitis. The prognosis is poor with frequent and severe functional sequelae. Treatment is mainly based on high-dose systemic corticotherapy alone or combined with cyclophosphamide. Our patient responded favourably to early methylprednisolone bolus followed by oral corticosteroids and antimalarial treatment. CONCLUSION: Lupus profundus is classically of benign course with only cutaneous involvement, but it may sometimes be a sign of systemic lupus and in rare cases may be associated with severe complications.
Abstract: The spectrum of diseases found in series of fever of unknown origin shows variation in relation to selection bias; particularly, selection of the most difficult cases in tertiary reference university centres. We present a series of 144 patients presenting to a non-university hospital between 1999 and 2005 (secondary level of the health care system) with a community-acquired fever of unknown origin. In 37 cases (25.7%), the reason for fever could not be explained. Among the 107 patients with a final diagnosis (74.3%), non-infectious inflammatory disorders represented the most prevalent category (35.5%), surpassing infections (30.8%), miscellaneous causes (20.6%) and malignancies (13.5%). 13 entities accounted for over 68% of diagnoses (sinusitis and occult dental infections, Q fever, Epstein-Barr virus and cytomegalovirus infections, lymphoma, colo-rectal adenocarcinoma, adult-onset Still disease, systemic lupus erythematosus, giant cell arteritis and/or polymyalgia rheumatica, rheumatoid arthritis, polyarteritis nodosa, factitious fever and habitual hyperthermia). As demonstrated in other studies, non-infectious inflammatory diseases emerge as the most prevalent diagnostic category. Giant cell arteritis and polymyalgia rheumatica were particularly frequent in the elderly. Epstein-Barr virus and cytomegalovirus infections and habitual hyperthermia were particularly frequent in the youngest patients. There were no major differences in repartition of diagnostic categories between this series and historical university series.
Abstract: INTRODUCTION: Idiopathic hypereosinophilic syndrome is defined by the combination of persistent eosinophilia and evidence of tissue damage. It is a diagnosis of exclusion. CASE: We report the case of a 53-year-old man with idiopathic hypereosinophilic syndrome associated with an orbital inflammatory pseudotumor. Therapy with corticosteroids rapidly decreased the number of circulating eosinophiles; the orbital inflammatory pseudotumor regressed at the same time. DISCUSSION: This is the first report of idiopathic hypereosinophilic syndrome associated with an orbital inflammatory pseudotumor.
Abstract: INTRODUCTION: Sarcoidosis is a multisystem disorder that rarely involves the genitourinary tract. CASE: A 35-year-old man had intrascrotal extratesticular mass. Surgical exploration found noncaseating granulomatous inflammation. After the CT scan revealed mediastinal lymphadenopathy and splenomegaly, we diagnosed sarcoidosis. The patient responded rapidly to corticosteroids. DISCUSSION: Intrascrotal sarcoidosis is an unusual differential diagnosis for an intrascrotal mass. Histological testing is necessary to diagnose noncaseating granulomas. Other granulomatous diseases must be ruled out. Corticosteroid treatment is indicated only for symptomatic sarcoidosis with diffusion of granulomatous lesions and involvement of vital organs.
Abstract: We report the case of a patient who developed brain abscesses caused by Abiotrophia defectiva. The patient was treated with prednisone and cyclophosphamide for connective-tissue disease (lupus-Sjögren's overlap syndrome). A few cases of central nervous system infections due to Abiotrophia species have been previously reported in immunocompetent patients following neurosurgical procedures, suggesting possible introduction of this organism at the time of surgery. This case report is the first report of brain abscesses due to A. defectiva in a pharmacologically immunosuppressed patient.
Abstract: INTRODUCTION: Aortitis consists in aortic wall inflammation from infectious or non infectious cause. It may lead to aortic aneurysm with a risk of rupture, which is life-threatening and may justify surgical procedures. The cause of the aortitis is sometimes obscure. CASE REPORT: We report the case of a 55 years old woman who developed acute aortitis of the descending aorta after G-CSF (granulocyte-colony stimulating factor) injections for blood stem cells graft. No cause was found to the aortitis, the evolution was favorable after corticosteroid treatment, without aneurysm at six months. CONCLUSION: The present case rises the question of G-CSF (Neupogen responsibility in aortic lesions. Neutrophilic mediated diseases (Sweet's syndrome, pyoderma gangrenosum) and leukocytoclastic vasculitis were reported after G-CSF therapy. Neutrophils induced by G-CSF injections present functional abnormalities which may play a role in the pathogenesis of these diseases.
Abstract: INTRODUCTION: Myelopathy is a rare manifestation of systemic lupus erythematosus, occurring most often during the course of the disease. EXEGESIS: We report two cases of women with myelopathy as the first manifestation of systemic lupus erythematosus; both had an unusual course. We review the literature for previously reported cases. CONCLUSION: The clinical presentation of myelitis is heterogeneous. Usually, neurologic deficits evolve within a few hours (typically acute transverse myelitis) and outcome is usually poor. However, chronic or recurrent transverse myelitis has also been reported, including relapsing myelitis that resolved spontaneously. Myelopathy can be the first manifestation of the disease and this might be more common than initially thought. Magnetic resonance imaging (MRI) findings depend on the timing of the examination and the stage of the disease; the MRI may therefore be normal. An association with optic neuritis is frequently reported in the literature and differential diagnosis with multiple sclerosis may be difficult. Overlapping features between both diseases have been termed "lupoid sclerosis" and are actually classified as demyelinating syndromes associated with lupus. Myelopathy does not appear to be consistently associated with antiphospholipid antibodies, as has been previously suggested. The best treatment protocol has not been determined; however, in recent years, pulses of methylprednisolone and cyclophosphamide have gained acceptance by most authors.
Abstract: BACKGROUND: Anti oestrogenic treatment is widely used for breast cancer treatment and prevention of recurrence. Because of concomitant estrogenic effects, tamoxifen exerts carcinogenic properties on the endometrium. Although secondary endometrial cancers usually present as pure adenocarcinomas, other types of rare tumors have also been reported. PATIENTS AND METHODS: Herein we describe the clinical, pathological as well as therapeutic aspects of a new case of endometrial mesodermal mixed tumor occurring after long-term tamoxifen therapy. RESULTS: The present case occured five years after cessation of a five years tamoxifen treatment. The patient failed to respond to doxorubicin and cyclophosphamide when combined to 5-fluorouracil (5-FU), but she reached complete response when the same two drugs were used with carboplatin, suggesting the potential usefullness of platinum derivatives. CONCLUSIONS: A longer latency period might be observed for endometrial mesodermal mixed tumors as compared to adenocarcinomas and could justify a prolonged clinical and ultrasonographic follow-up of patients during and after tamoxifen treatment. When indicated, chemotherapy might require the use of platinum derivatives in this particular type of secondary tumor.
Abstract: The clinical characteristics, outcome and treatment of non-tuberculous mycobacterial tenosynovitis are reviewed. From lesions localized in the hand, 10 different species of non-tuberculous mycobacteria have been reported. The most common are Mycobacterium marinum and Mycobacterium kansasii. Other less frequent organisms are Mycobacterium avium complex, Mycobacterium szulgai, Mycobacterium terrae, Mycobacterium fortuitum, Mycobacterium chelonae, Mycobacterium abscessus, Mycobacterium malmoense and Mycobacterium xenopi. The infections appear to be the result of previous trauma, surgical procedure, corticosteroid injection or non-apparent inoculation (water contamination). Immunosuppression is sometimes associated with the infections and can be considered as a risk factor. Surgical debridement and appropriate mycobacterial cultures are critical to enable diagnosis and appropriate management. Specimens should be inoculated on a range of media and incubated at a range of temperatures in order to isolate mycobacteria with different growth characteristics (with prolonged incubation). The optimal treatment of these infections is discussed.
Abstract: INTRODUCTION: Constrictive bronchiolitis obliterans is defined histologically as obliteration of the lumina of bronchioles by inflammatory tissue elements and progressive destruction of bronchioles, eventually replaced by fibrotic tissue. Most of the cases, which are not associated with either solid organ or bone marrow transplantation, occur in the development of rheumatoid arthritis. EXEGESIS: We present the case of a 73-year-old woman who developed severe constrictive bronchiolitis obliterans without readily identifiable cause, rapidly fatal despite corticosteroid therapy. The patient had antinuclear antibodies (titer 1/1024) and antiphospholipid antibodies, but there was no anti-DNA, anti-ENA or anti-beta 2GPI antibodies. Tests for rheumatoid factor were negative. Cryoglobulinemia was present (monoclonal IgM Kappa). CONCLUSION: In this case, constrictive bronchiolitis obliterans was associated with serologic abnormalities and cryoglobulinemia. However, were was no clinical evidence of connective tissue disease.
Abstract: INTRODUCTION: Necrobiotic xanthogranulomatosis is a rare cutaneous disorder usually associated with monoclonal gammopathy. Most frequently, cutaneous lesions involve the face (periorbital region) and the trunk, and are characterized by indurated xanthomatous plaques and nodules. EXEGESIS: We describe a patient presenting with necrobiotic xanthogranulomatosis that was unusual, as it was associated with IgM kappa monoclonal gammopathy of undetermined clinical significance and because no periorbital involvement was observed. CONCLUSIONS: Necrobiotic xanthogranulomatosis should be distinguished from other cutaneous manifestations associated with plasma cell dyscrasias, such as normolipemic plane xanthoma. It can be associated with IgM gammopathy.
Abstract: The occurrence of cancers in patients with primary humoral immunodeficiency syndrome (X-linked agammaglobulinemia, common variable immunodeficiency, IgA deficiency) is more than mere coincidence. An increased risk of non Hodgkin's lymphoma and gastric adenocarcinoma, particularly for patients with common variable immunodeficiency, is well established. A literature review is presented on this subject with analysis of case reports, prospective and retrospective studies, and data from the Minnesota Immunodeficiency Cancer Registry.
Abstract: Mediterranean spotted fever is a tick-borne disease that is endemic in the Mediterranean basin from spring to autumn. Usually mild, the disease can be severe in some cases, especially when risk factors are encountered in patients or when treatment is delayed. The correlation between these malignant forms and patients' immunological disorders remains unclear, while the pathophysiology of the disease seems well known. A case of a malignant form of Mediterranean spotted fever is reported which occurred 2 months prior to the diagnosis of polymyalgia rheumatica. Evidence of immunological disorders consisted only in an antiphospholipid antibody associated with a transient lupus anticoagulant. No underlying risk factors other than the primary undiagnosed phase of polymyalgia rheumatica has been observed.
Abstract: We report the case of a 75-year old woman presenting sarcoid myopathy with pseudohypertrophy and skin involvement. Muscular biopsy confirmed the diagnosis of sarcoidosis. Symptomatic muscle involvement in sarcoidosis is rare. Three forms are described: myopathic, myositic and nodular. The relevance of imaging techniques is reviewed. Treatment is based on corticotherapy which is less efficient in myopathic form and in this case we had to use methotrexate with success.
Abstract: The case of a 22-year-old man with polyneuropathy, endocrinopathy, skin change and monoclonal gammopathy of IgG-lambda type is described. There was no solitary plasmocytoma, osteosclerotic myeloma or Castleman's disease. However, significant thrombocytosis occurred and the patient developed arterial thrombosis, these were attributed to essential thrombocythaemia in the absence of other aetiological factors.
Abstract: Usually, presenting infections in children with agammaglobulinemia include pneumonia and otitis media caused by pyogenic bacteria. We report here two cases of Pseudomonas aeruginosa septicemia with ecthyma gangrenosum, in previously healthy boys, leading to the diagnosis of X-linked agammaglobulinemia.
Abstract: Rifabutin is used in patients with human immunodeficiency virus infection to prevent and treat Mycobacterium avium complex infection. We report a case of recurrent bilateral anterior uveitis with hypopyon in a patient who was taking 600 mg of rifabutin daily. The rate of recurrence with the continuation of rifabutin seems to be high, especially in the opposite eye (alternate uveitis with hypopyon); rifabutin should be discontinued if uveitis recurs.
Abstract: Cases of cancer have been reported in patients with primary humoral immunodeficiency syndromes, mainly non-Hodgkin's lymphoma and gastric adenocarcinoma. Two cases of Hodgkin's disease complicating IgA and IgG subclass deficiency are reported. This association is probably more than mere coincidence. A review of the literature is presented.
Abstract: The major manifestations of giant cell arteritis have been well described. Pulmonary manifestations, however, are rare. We report the case of a 75 year old woman with temporal arteritis, presenting with atypical manifestations, i.e. nodular pulmonary lesions, dry cough, rhinitis, conjunctivitis, and otitis with hearing loss. We conclude that overlapping features of giant cell arteritis and Wegener's granulomatosis occur in some patients.
Abstract: The authors report two cases of Lambert-Eaton myasthenic syndrome associated with small cell lung carcinoma. Following the observations, the clinical diagnosis of this syndrome is considered. We discuss the autoimmune pathogenesis and the relation between paraneoplastic syndrome and small cell cancer. This syndrome is caused by autoantibodies that block the voltage-dependent calcium channels at motor nerve terminals. Small cell carcinoma cells appear to express calcium channels, suggesting that autoantibody production may be triggered by tumor calcium channels determinants. The autoimmune paraneoplastic syndrome theory refers to cross-antigenicity.
Abstract: Pulmonary disease is an uncommon extraintestinal manifestation of inflammatory bowel disease. We report the case of a patient in whom colectomy for ulcerative colitis was followed by development of bronchiectasis. A discussion of the relation between ulcerative colitis and bronchial disease is presented.
Abstract: Paraneoplastic neurological syndromes are mostly associated with small cell lung cancer. Lambert-Eaton myasthenic syndrome appears to be caused by anti-presynaptic calcium channel antibodies. Calcium channels are also present in the cell membrane of small cell lung cancer, which may trigger the formation of anti-calcium channel antibodies. It is the most convincing argument in support of the auto-immune paraneoplastic theory, which refers to cross-antigenicity. Serum of patients with small cell carcinoma and cancer-associated retinopathy contains immunoglobulins against several antigens in the retinal and tumor cells. Patients with chronic intestinal pseudoobstruction (gastrointestinal neuropathy) associated with small cell lung cancer displayed circulating IgG antibodies reactive with neurons of myenteric plexus (anti-enteric neuronal antibodies). On the other hand, high levels of anti-neuronal antibodies (anti-Hu) have been found in the serum and cerebrospinal fluid of patients suffering from subacute encephalomyelitis (limbic encephalitis, cerebellar degeneration, sensory neuronopathy) associated with small cell lung cancer. The pathogenic role of the anti-neuronal antibody is not well established. Nevertheless, the finding of high titer antineuronal antibody in patients with a suggestive clinical syndrome is of great interest since it confirms the paraneoplastic syndrome and suggests the location of the primary tumor when the cancer is unknown.
Abstract: Between April 1982 and June 1989, 65 patients (15 T1, 13 T2, 32 T3, 5 T4) with squamous cell carcinoma of the oesophagus were treated with a curative intent with multimodality combined treatment. A first course of 5-fluorouracil and cisplatin was given during work up, especially if NdYAG laser therapy was used. Irradiation was started 3-4 weeks after induction and two courses of concomitant chemotherapy were given during the radio therapy (aiming at 64 Gy over 7 weeks). Actuarial survival was 79.6% at 1, 36.7% at 3 and 26.7% at 5 years. 5 year survival rates were 56.3% for T1, 29.8% for T2 and 12.9% for T3. All T4 cases died within 16 months. Complete initial disease response was achieved in 76%. Tolerance was good. Thus patients with squamous cell carcinoma of the oesophagus can have long survival and may be cured with combined modality therapy. This treatment may be an alternative to radical surgery when there is a high risk of operative mortality.
Abstract: The Eaton-Lambert syndrome is a clinical and electrophysiological entity, which affects less than 3% of patients with a small cell cancer. In the majority of cases the syndrome is present before the discovery of cancer. The diagnosis is primarily suggested by proximal muscle fatiquibility in the lower limbs. The confirmation of the diagnosis is carried out by an electromyography. The occurrence of non-para neoplastic cases are frequently associated with disorders of immunity (Auto-immune Disease, and various auto-antibodies) in the groups HLA B8 and DR3. The efficacy of immunosuppressants has led to a search for an auto-immune mechanism as the basis for this myasthenic type syndrome. The demonstration in recent years of anti-calcium channel antibodies at the origin of the pre-synaptic junctional block has confirmed this hypothesis. The demonstration of the auto-immune character of this para-neoplastic syndrome has led to better understanding of the host tumour relationship from an immunopathological stand point.
